Paget disease is a localized disorder of bone remodelling that causes bones become brittle and crooked. This disorder typically begins with excessive bone resorption by osteoclast followed by an increase in bone formation by osteoblast.

This excessive osteoclastic activity followed by compensatory activity of osteoblasts leading to a structurally disorganized mosaic of bone (woven bone), which is mechanically weaker, larger, less compact, more vascular, and more prone to fracture than normal adult lamellar bone. This disease is localized to one particular part or several parts of the body, and most often occurs in the area of the pelvis, skull, spine and leg bones.

In 1877, the British surgeon Sir James Paget first described chronic inflammation of the bone as osteitis deformans, then it became the second most common bone disorder, after osteoporosis, in the elderly.

Around 70-90% of people with Paget disease are asymptomatic, however, some patients may experience the following symptoms:

  1. Bone pain is the most common symptom
  2. Secondary osteoarthritis when Paget disease occurs around the joints
  3. Bone deformities that most often bending of the extremities
  4. Excessive warmth due to hypervascularity
  5. Neurological complications caused by compression of nerve tissues
  6. Headaches to hearing loss caused by disorder that occurs in skull bone area
  7. Tingling, numbness in the arms and legs, back and neck pain caused by disorders that occurs in spine area

Paget disease is common in Europe, North America, Australia, and New Zealand. It is rare in Asia and Africa. Paget disease will increase with age, estimated to occur in 1-3% of individuals older than 45-55 years and up to 10% in those aged >80 years. The highest prevalence of pelvic Paget disease in individuals over 65 years of age. This disease is more common in men than women.

Although the cause of this disease is still unknown, there are genetic and environmental factors that could increase risk factor of Paget disease. Environmental factors, namely viral infection, exposure to pollution such as dust, air or chemicals. Other possible causes are autoimmune, connective tissue and blood vessels disorders.

Initial diagnosis conducted with anamnesis and physical examination, then proceed with diagnostic tests, such as:

  • X-rays shows the enlarged and thickened bone or bowing of an extremity.
  • Bone scan shows detail of the bone that affected by Paget's disease.
  • Blood test to determine alkaline phosphatase levels. Typically, people with Paget's disease have higher levels of alkaline phosphatase.
  • Bone biopsy for Paget's disease confirmation which performed by taking a sample of bone cells for further examination in a laboratory.

Complications of Paget disease, such as:

  1. Bones fractures
  2. Osteoarthritis due to pressure around the joint area that has bone deformities
  3. Walking difficulties due to bony deformity
  4. Hypercalcemia causes by increasing bone breakdown that impact blood calcium levels
  5. Nerve disorders, further will cause tingling in the hands or feet and loss of hearing ability
  6. Heart failure due to an increase of heart workload to the body areas that affected by Paget disease
  7. Bone Cancer that can occur in 1% of the patient population with Paget disease

The short-term objective of Paget disease treatment is to control disease activity. The long-term objectives of treatment are to minimize or prevent disease progression and to decrease complications from the disease, if possible. The drug treatment of Paget disease are as follows:

  • Bisphosphonates to inhibit overactivity of osteoclasts
  • Calcitonin to regulate calcium levels and bone metabolism. This drug is only given if the patient is not compatible with bisphosphonate drugs
  • Nonsteroidal anti-inflammatory drugs (NSAIDs), such as ibuprofen or diclofenac as pain reliever

 

 

Surgery procedures could be performed to help with the fractured bone, improve the position of the bone, reduce pressure on nerves, or replace damaged joints. The following are the surgical procedures that can be performed:

  • Internal fixation (pen surgery), adjusting bones in proper position.
  • Osteotomy which is a bone surgery procedure to remove damaged bone cells, relieving pain, and correction to the position of bones and joints.
  • Joint replacement with an artificial joint (prosthesis) made of metal, plastic, or ceramic.

Prevention for Paget's disease can be done by maintaining bone health, joint movement (mobility), meeting calcium and vitamin D intake, and routine exercise. When you have been diagnosed with this disease, do regular check-ups on a regular basis, follow the recommendations and treatment given by the physician.

 

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